Surgical Treatment of Craniosynostosis: Outcome Analysis of 250Consecutive Patients

Gerald M. Sloan, MD*; Karin C. Wells, MD‡; Corey Raffel, MD, PhD§; and J. Gordon McComb, MD‡

ABSTRACT. Objective. Surgery for craniosynostosishas evolved rapidly over the past two decades, withincreased emphasis on early, extensive operations. Olderpublished series may not accurately reflect more recentexperience. Our study was designed to analyze outcomein a large series of consecutive patients treated recently ata single center.

Methods. We reviewed 250 consecutive patients whounderwent surgical treatment of craniosynostosis be-tween January 1, 1987 and December 31, 1992. They weredivided into nine groups by suture involvement: sagittal,unilateral coronal, bilateral coronal, unilateral lambdoid,bilateral lambdoid, metopic, multiple suture, the Klee-blattschadel deformity (cloverleaf skull), and acquiredcraniosynostosis. Outcome was analyzed in terms of re-sidual deformities and irregularities, complications, mor-tality, as well as the need for additional surgery.

Results. There were 157 males (62.8%) and 93 females(37.2%), with most of the male preponderance accountedfor by the large sagittal synostosis group, which con-sisted of 82 males and 25 females. Median age at firstoperation was 147 days. A named syndrome was presentin 23 patients (9.2%) and was more common than ex-pected with bilateral and unilateral coronal synostosis,the Kleeblattschadel deformity, and multiple suture syn-ostosis. There were two deaths (0.8%), both with Klee-blattschadel patients, and 17 other complications (6.8%).Morbidity and mortality were significantly associatedwith secondary vs primary operations and syndromic vsnonsyndromic patients. Outcome analysis revealed thebest surgical results with metopic synostosis and signif-icantly less good results with the Kleeblattschadel defor-mity, multiple suture synostosis, and bilateral coronalsynostosis.

Conclusions. Using modern surgical techniques, cra-niosynostosis can be corrected with good outcomes andrelatively low morbidity and mortality, particularly forotherwise healthy, nonsyndromic infants. Pediatrics1997;100(1). URL: http://www.pediatrics.org/cgi/content/full/100/1/e2; craniosynostosis, craniofacial anomalies,craniofacial surgery, facial deformities.

Surgery for craniosynostosis has evolved rapidlyover the past two decades, with increased em-phasis on early, extensive operations.1–8 Because

of changes in surgical timing and techniques, earlierseries may not accurately reflect more recent experi-ence. Furthermore, most previous reports do notquantitate results in a way that would allow objec-tive analysis and comparisons. An exception is thefour-category classification of operative results intro-duced by Whitaker et al,6 which has subsequentlybeen used by others.7,8 In that classification system,category I includes those patients in whom no sur-gical revisions were considered advisable or neces-sary by the surgeon, patient, or family. In category II,soft tissue or minor bone contouring revisions weredesirable, whether or not they were actually per-formed. Category III consisted of patients in whommajor secondary osteotomies or bone grafting proce-dures were needed or performed. These procedureswere not as extensive as the original surgery. Cate-gory IV was composed of those patients in whom amajor craniofacial procedure, duplicating or exceed-ing the extent of the original surgery, was or wouldbe necessary. However, that classification system hassignificant limitations. Categories II, III, and IV allrepresent patients believed to require further sur-gery. Good or fair results, with residual deformities,but not requiring further surgery, are not distin-guished from excellent results.

To examine results of recent craniosynostosis sur-gery, we analyzed the outcome in 250 consecutivepatients treated at a single center during a 6-yearperiod beginning January 1, 1987 and ending Decem-ber 31, 1992. We developed a seven-category out-come classification system (Table 1) to allow recog-nition of more subtle differences in surgical results.The patient numbers are sufficient to permit detailedstatistical analysis of results for a well-defined groupof patients. With increasing emphasis on clinical out-come analysis at the national level, such data will beincreasingly important in developing multidisci-plinary treatment protocols.

MATERIALS AND METHODSA total of 260 patients underwent a surgical procedure for

craniosynostosis at Childrens Hospital Los Angeles between Jan-uary 1, 1987 and December 31, 1992. Seven patients were excludedbecause their first operation for craniosynostosis had been per-formed before January 1, 1987. Three other patients were excludedbecause their first operation, although falling within the 6-yearstudy period, was performed elsewhere. The remaining 250 pa-tients had undergone initial surgical treatment of craniosynostosis

From the *Division of Plastic Surgery, University of North Carolina Schoolof Medicine, the §Department of Neurosurgery, Mayo Clinic and Founda-tion, Rochester, Minnesota and the ‡Divisions of Plastic Surgery and Neu-rosurgery, Childrens Hospital Los Angeles, Los Angeles, California.Presented in part at the 73rd annual meeting of the American Association ofPlastic Surgeons, St Louis, MO, May 1–4, 1994.Received for publication Mar 13, 1996; accepted Oct 8, 1996.Reprint requests to (G.M.S.) Professor and Chief, Division of Plastic andReconstructive Surgery and Surgery of the Hand, University of NorthCarolina School of Medicine, Chapel Hill, NC 27599–7195.PEDIATRICS (ISSN 0031 4005). Copyright © 1997 by the American Acad-emy of Pediatrics.

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at Childrens Hospital Los Angeles between January 1, 1987 andDecember 31, 1992.

The medical records of the 250 patients were reviewed. Datawere collected, including name, medical record number, date ofbirth, gender, involved sutures, other medical diagnoses, dates ofall surgical procedures performed for craniosynostosis, complica-tions, dates of follow-up visits, findings at follow-up, and the mostrecent assessment of outcome. Any other relevant data were alsonoted.

Data were analyzed for the entire patient group, as well as fornine subgroups based on suture involvement: sagittal, unilateralcoronal, bilateral coronal, unilateral lambdoid, bilateral lambdoid,metopic, multiple suture, Kleeblattschadel, and acquired. TheKleeblattschadel group consisted of those patients with the classiccloverleaf skull and total sutural synostosis. The multiple suturesynostosis group was defined as those patients with synostosis ofmore than one suture, excluding bilateral coronal, bilateral lamb-doid, and Kleeblattschadel patients.

To be able to analyze surgical results, we developed a seven-category classification system (Table 1). In this system, classes 1through 4 represent good to excellent overall correction of thedeformity, but with varying degrees of minor visible and/orpalpable irregularities: none in class 1, palpable but not visibleirregularities in class 2, visible irregularities in class 3, and requir-ing reoperation in class 4. Examples would include a palpable butnot visible surgical wire in the temporal region (class 2), a visibleand palpable bone spicule in the forehead (class 3), or a visible andpalpable surgical plate requiring surgical removal because of im-pending exposure (class 4). Classes 5 through 7 represent patientswith significantly compromised correction: not requiring furthersurgery in class 5, requiring further surgery in class 6, and be-lieved by the surgeon to require further surgery but with thefamily declining further surgery in class 7. Examples would in-clude noticeable brow asymmetry after correction of unilateralcoronal synostosis but not severe enough for reoperation (class 5),or residual brachycephaly after surgery for bilateral coronal syn-ostosis requiring repeat frontal-orbital advancement (class 6).

RESULTS

Suture InvolvementDistribution of patients by suture involvement is

shown in Table 2. By far the largest group was sag-

ittal synostosis, 107 patients (42.8%). Next most fre-quent were multiple suture synostosis (12.0%), uni-lateral lambdoid synostosis (12.0%), and unilateralcoronal synostosis (11.2%).

GenderGender distribution, presence of an identified syn-

drome, and median age at first operation are shownin Table 3. There was a clear male preponderanceamong patients with sagittal synostosis (76.6%) (P ,.0001). However, the bilateral coronal synostosis pa-tients were 76.2% female (P , .05). Taking the entiregroup of 250 craniosynostosis patients, there were 64more males. Almost the entire difference can be ex-plained by the sagittal synostosis group, which had57 more males than females.

Syndrome DiagnosisMore than half of the bilateral coronal synostosis

patients, 12 of 21, or 57.1%, carried the diagnosis of aspecific named syndrome (P , .001). Six of thetwelve had Crouzon, four Apert, one Antley-Bixler,and one Pfeiffer syndrome. A named syndrome waspresent in 4 of 30 multiple suture synostosis patientsor 13.3% (P , .001), with three having Crouzon andone Apert syndrome. Two of the six Kleeblattschadelpatients, or 33.3% (P , .01), carried a syndromediagnosis, one Antley-Bixler and one Pfeiffer. Twounilateral coronal synostosis patients had syndromediagnoses, one Baller-Gerold and one Saethre-Chot-zen. Additionally, there was one metopic synostosispatient with Klippel-Feil syndrome, one unilaterallambdoid synostosis patient was a 47 xxx female,and one sagittal synostosis patient had the Golden-har variant of the facio-auriculo-vertebral spectrum(Fig 1).

It is striking that the Kleeblattschadel patientswere operated on at a much earlier age (median 25days) than any other group. Because of the totalsutural involvement and high risk of resultant dam-age to the central nervous system from increasedintracranial pressure, these patients were treatedquite urgently. The sagittal synostosis group was thenext youngest group at the time of initial surgery(median 101 days). Many of those patients weretreated by sagittal craniectomy using the techniquethat has been described by McComb.9 Some of theyounger (less than 6 months) patients in the saggittalsynostosis group had a strip craniectomy. Some of

TABLE 1. Classification of Surgical Result After Reconstruction for Craniosynostosis

Class 1 Good to excellent correction, with no visible or palpable irregularityClass 2 Good to excellent correction with palpable but not visible irregularity (eg, a palpable, but not

visible, surgical wire, plate, or bony irregularity), not requiring reoperationClass 3 Good to excellent correction with visible irregularity (eg, a visible prominence from a surgical

wire or plate, or a visible bony spicule or defect that does not compromise the overallcorrection), not requiring reoperation

Class 4 Good to excellent correction with visible or palpable irregularity requiring reoperation (eg, asurgical plate requiring removal)

Class 5 Compromised overall correction, but not severe enough to require reoperation (eg, slightforehead asymmetry)

Class 6 Compromised overall correction requiring reoperationClass 7 Compromised overall correction, believed to require reoperation by the surgeon, but family

declines further surgery

TABLE 2. Suture Involvement for 250 Consecutive SurgicalPatients With Craniosynostosis

Sutural Involvement No. % of Total

Sagittal 107 42.8Unilateral coronal 28 11.2Bilateral coronal 21 8.4Unilateral lambdoid 30 12.0Bilateral lambdoid 7 2.8Metopic 20 8.0Multiple suture 30 12.0Kleeblattschadel 6 2.4Acquired 1 0.4

Total 250 100.0

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the older patients underwent the p or reverse pprocedure.10

ComplicationsComplications, deaths, and number of patients un-

dergoing planned as well as unplanned reoperationare shown in Table 4. The only two deaths, as well astwo nonfatal complications, occurred in the Klee-blattschadel group. One of the deaths was a maleinfant with Antley-Bixler syndrome, who underwentradical posterior craniectomy at 9 days, followed bybilateral frontal-orbital advancement and remodel-ing at 18 days. He subsequently developed turri-cephaly, for which barrel stave osteotomies wereperformed at 16 months. He died, shortly after thatsurgery, of acute brain herniation. The second death

was a male infant with Pfeiffer syndrome who un-derwent posterior craniectomy at 25 days, which wascomplicated by an intraoperative dural sinus hemor-rhage requiring transfusion of 2 units of packed redblood cells and 40 units of platelets. At 3 months,bilateral frontal orbital advancement and remodelingwas performed, which was followed by postopera-tive hydrocephalus, requiring ventriculo-peritonealshunting 2 months later. On follow-up examinationat 14 months, there was found to be no evidence ofhead growth since the shunt placement. The infantwas developmentally delayed. Posterior vault re-modeling was performed and was complicated byvenous sinus hemorrhage. This was controlled intra-operatively, but the patient died of cerebral edema 3days later.

Fig 1. A, B, and C, Three views of a 1-month-old male infant with multiple congenital anomalies including sagittal stenosis, bilateral cleftlip and palate, and right lateral facial cleft. He carries the diagnosis of facio-auriculo-vertebral spectrum. D, The same patient at 10 months,after sagittal craniectomy at 2 months through midline incision, and calvarial vault remodeling at 9 months. E, The same patient at 5 years.

TABLE 3. Gender Distribution, Identifiable Syndromes, and Median Age at First Operation for 250 Consecutive Surgical PatientsWith Craniosynostosis, by Sutural Involvement*

Sutural Involvement No. Male(% of group)

No. Female(% of group)

No. Syndromic(% of group)

Median Age At FirstOperation (in days)

Sagittal †82 (76.6) †25 (23.4) 1 (0.9) 101

Unilateral Coronal 13 (46.4) 15 (53.6) ‡2 (7.1) 198

Bilateral Coronal ‡5 (23.8) ‡16 (76.2) §12 (57.1) 136

Unilateral Lambdoid 19 (63.3) 11 (36.7) 1 (3.3) 196

Bilateral Lambdoid 6 (85.7) 1 (14.3) 0 203

Metopic 11 (55) 9 (45) 1 (5) 195

Multiple Suture 17 (56.7) 13 (43.3) §4 (13.3) 138

Kleeblattschadel 4 (66.7) 2 (33.3) \2 (33.3) 25

Acquired 0 1 (100) 0 719

Total 157 (62.8) 93 (37.2) 23 (9.2) 147

* P values compared to expected 50–50 gender distribution and 0.7% rate of named syndromes, based on binomial (n # 20) or Poisson(n . 20) distributions.† P , .0001.‡ P , .05.§ P , .001.\ P , .0001.

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Five of 21 bilateral coronal synostosis patients(23.8%, P , .001) had complications, which includedtongue edema requiring reintubation, a dural andcortical laceration resulting in a postoperative cere-brospinal fluid leak that resolved, femoral arterythrombosis at a catheter site, a subdural fluid collec-tion that required shunting, and a pyogenic granu-loma at the surgical incision. Four of 30 multiplesutures synostosis patients (13.3%, P , .05) had com-plications: postoperative apnea requiring reintuba-tion, postoperative seizures, severe conjunctivaledema and herniation, and superficial stitch ab-scesses along the incision. Two of 30 unilateral lamb-doid synostosis patients (6.7%, P , .05) had compli-

cations: a unilateral supranuclear facial palsy thatresolved 8 months after surgery, and Salmonella sep-sis in a patient whose father had a diarrheal illnessshortly before surgery. Four of 107 sagittal synostosispatients (3.7%, P , .001) had complications: pneu-monia, postoperative hydrocephalus requiringshunting, intraoperative metabolic alkalosis, and asuperficial wound infection.

Unplanned reoperation was required in 18 of the250 patients (7.2%). Specifically, unplanned reopera-tion was needed in two Kleeblattschadel (33.3%),four bilateral coronal (19.0%), five multiple suture(16.7%), two unilateral coronal (7.1%), two unilaterallambdoid (6.7%), one metopic (5%), and two sagittalsynostosis patients (1.9%).

There were 17 nonfatal complications and 2deaths, a combined morbidity and mortality rate of19 of 250 patients (7.6%) or 19 of 297 operations(6.4%). For 250 primary operations, there were 10complications and no deaths (a complication rate of4.0%). For 47 secondary or tertiary operations, therewere two deaths and seven other complications, for acomplication rate of 14.9% (P , .01 compared toprimary operations by x2 analysis), and a combinedmorbidity and mortality rate of 19.1% (P , .001).

Analyzing morbidity and mortality by the pres-ence of an associated syndrome, it is striking that

Fig 3. A, 1-year-old male child with severe plagiocephaly second-ary to right coronal and right lambdoid synostosis. B, He under-went posterior craniectomy at 13 months and bilateral frontal-orbital remodeling at 15 months. He is shown here at 3 years. Hestill has lateral displacement of the cranial base on the right side,but this has not been severe enough to recommend further sur-gery, making this a class 5 result.

Fig 2. A, 2-month-old female infant with metopic, sagittal, andbilateral coronal synostosis. B, The same patient, on the operatingtable at 3 months, demonstrating the severe deformity. C, Intra-operative view demonstrating sagittal synostectomy and bilateralfrontal-orbital advancement and remodeling. D, The appearanceat the completion of surgery. E, The appearance at 22 months.

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both deaths and 9 of 17 nonfatal complications oc-curred in syndromic patients. Because our entire pa-tient group included 23 patients with syndrome di-agnoses, the mortality rate was 8.7% for syndromicpatients and 0% for nonsyndromic patients. The non-fatal complication rate was 39% for syndromic pa-tients as opposed to 3.5% for patients who did notcarry a syndrome diagnosis (P , .0001).

Multiple Suture SynostosisThe multiple suture synostosis group is a particu-

larly interesting and challenging group of patientsthat has not been well described elsewhere (Fig 2). Inthis group, we include all patients with synostosis oftwo or more cranial sutures other than isolated bi-lateral lambdoid synostosis, bilateral coronal synos-tosis, or the classic Kleeblattschadel (clover leaf skull)deformity, which three groups are classified sepa-rately. In our series, this group was surprisinglylarge, consisting of 30 patients, or 12% of the entirepopulation. Such a large number of unusual suturecombinations may reflect either increased awarenessand better ability to diagnose multiple suture in-volvement with modern imaging techniques, or aselection bias based on referral to a tertiary carecenter. The specific sutures involved for those 30patients are listed in Table 5. The most commoncombination was 10 patients with unilateral coronaland unilateral lambdoid synostosis. It is striking thatthe involvement of the two sutures was ipsilateral inall 10 patients; the right side for 7 patients and theleft for 3 patients. The resulting deformity was usu-ally quite severe (Fig 3). We have never seen a pa-tient with unilateral coronal and unilateral lambdoidsynostosis on opposite sides.

Acquired CraniosynostosisOne female infant in our series had acquired cra-

niosynostosis with congenital hydrocephalus whohad undergone ventriculo-peritoneal shunting anddeveloped fusion of multiple cranial sutures. This isa known, but uncommon, sequela of shunting that

has been previously described.12 Our patient wastreated with extensive calvarial remodeling withmultiple bone flaps at 23 months. She did well aftersurgery, but died 6 months later of unrelated sepsissecondary to necrotizing enterocolitis and bowel per-foration.

OutcomeOutcome after completion of planned surgery,

whether one or two operations, was analyzed ac-cording to the classification system listed in Table 1(Fig 4). Sagittal synostosis patients were not includedin this analysis, because they will be analyzed sepa-rately and reported elsewhere. All patients followedfor a minimum of 6 months after completion ofplanned surgery were included in this analysis. Theactual numbers are shown in Table 6, which includes115 of 143 possible patients. To allow statistical anal-ysis we assigned a numerical value to each class, asfollows: Class 1, 0 points; class 2, 0.5 point; class 3, 1point; class 4, 2 points; class 5, 3 points; class 6, 4points; and class 7, 4 points.

Although admittedly arbitrary, we attempted toassign numbers proportionate to the amount bywhich a result varied from class 1. Classes 6 and 7have the same numerical value because they were

TABLE 4. Complications, Planned and Unplanned Reoperations, and Deaths Among 250 Craniosynstosis Patients, by SuturalInvolvement*

SuturalInvolvement

Complications (%) Patients UndergoingPlanned Reoperation (%)

Patients RequiringUnplanned Reoperation (%)

Deaths

Sagittal †4 (3.7) 0 2 (1.9) 0

Unilateral Coronal 0 0 ‡2 (7.1) 0

Bilateral Coronal †5 (23.8) 5 (23.8) †4 (19.0) 0

Unilateral Lambdoid ‡2 (6.7) 0 ‡2 (6.7) 0

Bilateral Lambdoid 0 0 0 0

Metopic 0 0 1 (5) 0

Multiple suture ‡4 (13.3) 13 (43.3) †5 (16.7) 0

Kleeblattschadel ‡2 (33.3) 5 (83.3) §2 (33.3) 2 (33.3)

Acquired 0 0 0 0

Total 17 (6.8) 23 (9.2) 18 (7.2) 2 (0.8)

* P values compared to zero, for complications and for unplanned reoperations, based on binomial (n # 20) or Poisson (n . 20)distributions.† P , .001.‡ P , .05.§ P , .01.

TABLE 5. Distribution of Involved Sutures for 30 PatientsWith Multiple Suture Synostosis

Sutural Involvement No. % of Group

Unilateral coronal, unilateral lambdoid 10 33.3Metopic, sagittal 5 16.7Sagittal, unilateral coronal 2 6.7Sagittal, bilateral coronal 2 6.7Sagittal, unilateral lambdoid 2 6.7Unilateral coronal, bilateral lambdoid 2 6.7Metopic, unilateral coronal 1 3.3Metopic, unilateral lambdoid 1 3.3Metopic, sagittal, bilateral coronal 1 3.3Sagittal, unilateral coronal, bilateral lambdoid 1 3.3Sagittal, bilateral lambdoid 1 3.3Bilateral coronal, unilateral lambdoid 1 3.3Bilateral coronal, bilateral lambdoid 1 3.3

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believed to represent the same outcome, differingonly as to whether or not the family elected to havefurther surgery. The mean and SE of the outcomescores are listed in Table 7. The best outcome (lowestscore) was found in the metopic synostosis group

(Fig 5). Comparing the seven groups by the Tukeytest, looking for pair differences, significantly worseoutcomes were found for Kleeblattschadel, bilateralcoronal, and multiple suture synostosis patientscompared with metopic synostosis patients. No

Fig 5. A, 14-month-old male child with untreated metopic synostosis. B, The same patient on the operating table, before correction, at 17months. C, At the completion of surgery. D and E, At 2 years, 10 months.

Fig 4. A and B, 3-month-old female infant with left unilateral coronal synostosis. C and D, The same patient at 16 months, after bilateralfrontal-orbital remodeling at 5 months. She had three palpable stainless steel wires, above the hairline, which have not needed to beremoved. Therefore, this was a class 2 result.

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other statistically significant pair differences werefound.

DISCUSSIONWe found a striking male preponderance among

the sagittal synostosis patients; 82 males and 25 fe-males. That finding is consistent with the hypothesisof Graham et al,13 that fetal head constraint maycontribute to sagittal synostosis. The argument isthat the increased occurrence of sagittal synostosis inmales is related to larger fetal head size during thethird trimester of pregnancy, resulting in a higherdegree of physical constraint of the head in the ma-ternal pelvis. Overall, our surgical results with sag-ittal synostosis were excellent, with only four com-plications (3.7%) and only two unplannedreoperations (1.9%). The median age at surgery forthis group (a little more than 3 months) was lowerthan for all other groups except the Kleeblattschadelpatients.

The unilateral coronal synostosis patients had noperioperative complications, but a high proportion,46%, had a compromised aesthetic result because ofresidual postoperative asymmetry. In only one pa-tient was the asymmetry severe enough for reopera-tion to be recommended. However, that is a verysubjective decision. In this series, the median age atfirst operation for unilateral coronal synostosis wasalmost 7 months. We are presently operating onthese patients at a younger age (4 to 6 months) hop-ing to achieve better symmetry because the bone ismore pliable and the deformity is less severe at ayounger age.

The bilateral coronal synostosis group is strikingfor the preponderance of females (76.2%) and thefrequency of named syndromes (57.1%), the highestof any group in this study. Median age at first oper-

ation was 4.5 months. Five of 21 patients underwentplanned second operation, and 4 of 21 required anunplanned reoperation (P , .001). There was a highcomplication rate, 23.8% (P , .001), and all fivecomplications occurred in syndromic patients. Out-come was significantly worse than for our bestgroup, the metopic synostosis patients. Bilateralcoronal synostosis patients are a challenging group,at least partly due to the frequent presence of namedsyndromes with other associated problems and ab-normalities in these patients. Many, such as the Ap-ert and Crouzon patients, also have severe mid-facedeformities, making it difficult to achieve or evenjudge the proper amount of frontal and superiororbital advancement, whether done as the initial op-eration or as a combined monobloc advancement.14

There is still not a consensus as to the optimal timingand approach for these patients.8,15–19

The unilateral and bilateral lambdoid synostosispatients did well overall, although the unilaterallambdoid synostosis group did have a 6.7% compli-cation rate, and two patients required unplannedreoperation. As with the unilateral coronal patients,the major problem encountered with unilateral lamb-doid synostosis has been asymmetry. In fact, one ofour patients had such severe secondary foreheadasymmetry that she eventually underwent frontal-orbital remodeling after two occipital procedures.

Although unilateral lambdoid and bilateral lamb-doid synostosis patients comprised 12.0% and 2.8%of the patients in this series, respectively, that haschanged as of 1992. We are now operating on a farsmaller number of these patients. At present, whenwe have an opportunity to see patients before 1 year,we treat them with a molding headband20 (DynamicOrthotic Cranioplasty, Southwest Orthotic-Pros-thetic Laboratory, Phoenix, AZ). Initial results arevery encouraging and have been reported separate-ly.21 A recent report22 described a dramatic increaseas of 1992 in the number of infants seen at a singlecenter for plagiocephaly without synostosis. Theseinfants characteristically present with unilateral oc-cipital flattening that could be mistaken for unilaterallambdoid synostosis. We do not believe that any ofour unilateral lambdoid synostosis patients actuallyhad this entity, because radiographic and actual sur-gical findings were, in all our cases, consistent withtrue lambdoid synostosis. It has been hypothesizedthat plagiocephaly without synostosis has been seenwith increased frequency as of 1992 because that isthe year that the American Academy of Pediatrics

TABLE 6. Surgical Result After Reconstruction for Craniosynstosis in Patients Followed for a Minimum of 6 Months, Analyzed bySutural Involvement*

Class UnilateralCoronal

BilateralCoronal

UnilateralLambdoid

BilateralLambdoid

Metopic MultipleSuture

Kleeblattschadel

1 8 (30.8) 6 (31.6) 7 (36.8) 3 (60) 8 (50) 2 (7.7) 1 (25)2 3 (11.5) 1 (5.3) 3 (15.8) 0 6 (37.5) 7 (26.9) 03 2 (7.7) 3 (15.8) 6 (31.6) 1 (20) 1 (6.2) 5 (19.2) 04 1 (3.8) 0 0 0 1 (6.2) 2 (7.7) 05 11 (42.3) 5 (26.3) 1 (5.3) 1 (20) 0 6 (23.1) 06 1 (3.8) 4 (21.1) 2 (10.5) 0 0 3 (11.5) 2 (50)7 0 0 0 0 0 1 (3.8) 1 (25)

* Percentage of each group shown in parentheses; see Table 1 for definitions of classes 1 to 7.

TABLE 7. Numerical Outcome Scores for Patients After Sur-gical Treatment of Craniosynostosis, Ranked by Sutural Involve-ment, Minimum Follow-up 6 Months

Sutural Involvement No. Mean SE

Metopic 15 0.38 0.13Bilateral lambdoid 5 0.80 0.58Unilateral lambdoid 19 0.97 0.30Unilateral coronal 26 1.63 0.28Multiple suture 26 *1.79 0.28Bilateral coronal 19 *1.82 0.38Kleeblattschadel 4 *3.00 1.00

* Significant pair difference, compared to metopic synostosis pa-tients, by Tukey test.

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formally launched a campaign to educate the publicabout the association of the prone sleeping positionwith sudden infant death syndrome.23 An infant po-sitioned supine for sleep, it is suggested, may favorone side and thus expose that side to gentle butconstant pressure of enough magnitude to affecthead shape. Because that series ended in 1992, itseems unlikely that such factors played a role inmany, if any, of these cases.

Our best surgical results were obtained in themetopic synostosis patients (Table 7). We had nocomplications, performed no planned reoperations,and only one patient (5%) required unplanned reop-eration in the metopic synostosis group. A recentstudy, with 1-year follow-up CT measurements in 10metopic synostosis patients, demonstrated improvedbut persistent anterior orbital hypotelorism.24 Al-though our assessment was that these patients hadexcellent outcome overall, a number of these patientsdo have residual mild hypotelorism even after sur-gical correction.

The multiple suture synostosis patients were asurprisingly large and heterogeneous group. Four ofthe 30 patients had identified syndromes. There werehigh rates of complications (13.3%), planned two-stage correction (43.3%), as well as unplanned reop-erations (16.7%). It is difficult to generalize aboutsuch a diverse group, except to emphasize the needto individualize the treatment plan based on theinvolved sutures and the resulting deformity. If thereis significant lambdoid and/or coronal involvement,posterior release (craniectomy or cranioplasty) at ayoung age, perhaps 1 to 3 months, can relieve intra-cranial hypertension and allow waiting until 4 to 6months for the definitive frontal-orbital reconstruc-tion. By 4 to 6 months, we find the bone easier towork with to accomplish and stabilize frontal-orbitaladvancement and remodeling. The high incidence ofmultiple suture synostosis in our series, 12%, is strik-ing, particularly because bilateral coronal, bilaterallambdoid, and Kleeblattschadel patients are groupedseparately and make up an additional 13.6% in ourseries. Little has been written specifically about mul-tiple suture synostosis. Hoffman and Reddy25,26 re-ported the experience from the Hospital for SickChildren in Toronto, Canada, but what they de-scribed was a somewhat different entity. They found11 patients (1.7%) in a retrospective review of 665craniosynostosis patients over a 58-year period.However, their reports emphasized delayed and pro-gressive multiple suture synostosis, their patientswere older at presentation (24 to 117 months), mostof their cases were holocalvarial, and four of theircases followed previous surgery and might better beconsidered acquired craniosynostosis. Interestingly,Shillito and Matson,1 in their 1968 work based on 619surgical patients in a 40-year period at Children’sHospital Medical Center in Boston, reported 76 of the619 patients (14.6%) as having multiple suture syn-ostosis. Of those, 10 had involvement of two un-paired sutures, 36 had involvement of three sutures,and 30 had involvement of four or more sutures.Because that last group probably included patientsthat we have separately listed as Kleeblattschadel

deformity, it is striking how close their incidence,14.6%, is to the combined incidence of our multiplesuture patients and Kleeblattschadel patients, 14.4%.Also striking is that 38% of their multiple suturepatients were operated on more than once, in com-parison with 43.3% of our multiple suture patientswho underwent planned reoperation and 16.7% whorequired unplanned reoperation. Our rates of reop-eration for the six Kleeblattschadel patients wereeven higher.

The Kleeblattschadel deformity is characterized bya trilocular or cloverleaf cranial configuration, asso-ciated facial malformations, hydrocephalus, and (insome cases) micromyelia and skeletal anomalies.27

This has been a particularly difficult problem in ourexperience, as well as that of others. Despite plan-ning a two stage approach in five of our six patients,two of those patients still required additional un-planned operations. The only two deaths in our se-ries were those two patients.

Our one patient with acquired craniosynostosisdeveloped the problem after ventriculo-peritonealshunting for hydrocephalus. The presumed mecha-nism is rapid decompression of the cranial vault,after shunting, resulting in overlapping of the bonesacross what had been widely spaced sutures.12 Ifbrain growth does not soon spread the bones again,the sutures can become synostosed.

We found a striking association of mortality andmorbidity with the presence of a named syndrome.There are two possible explanations, both of whichmay play a role. First, 16 of the 23 syndrome patientshad bilateral coronal synostosis, multiple suture syn-ostosis, or Kleeblattschadel deformity, three groupsthat are particularly challenging surgically andwould have required longer, more extensive, andmore complicated surgical approaches. Second,many of the associated findings in the syndromicpatients, such as a retruded midface with airwaynarrowing, would put those patients at increasedrisk for complications.

In conclusion, we have reviewed a 6-year experi-ence with surgical management of craniosynostosisat a single center. This is the largest such series to beanalyzed and reported in almost 30 years. This reportprovides outcome data for craniosynostosis right upto the beginning of the recent controversies regard-ing the possible association of supine sleep position-ing and plagiocephaly without synostosis, an entitythat should be managed without surgery.22 Our re-sults demonstrate that true craniosynostosis can besuccessfully corrected, using modern surgical tech-niques, with relatively low morbidity (6.8%) andmortality (0.8%). Many unanswered questions re-main, but we hope that this report of a large, recentlytreated series of craniosynostosis patients will con-tribute to our evolving understanding and treatmentof these problems.

ACKNOWLEDGMENTSThis study was supported in part by RO1 DE10426 from the

Craniofacial Development and Disorders Program, National Insti-tute of Dental Research, National Institutes of Health.

The authors thank all individuals who rendered invaluable

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assistance in this study. Linda S. Chan, PhD and Carla Rother, MAperformed the statistical analysis. Susan E. Downey, MD, Larry S.Nichter, MD, and John F. Reinisch, MD participated in the sur-gery. Ari Blumoff, MD and Cathleen M. Salata, RN assisted withdata collection.

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Gerald M. Sloan, Karin C. Wells, Corey Raffel and J. Gordon McCombPatients

Surgical Treatment of Craniosynostosis: Outcome Analysis of 250 Consecutive

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